Table 2.
Summary of clinical course in patients with ACTH-producing thymic NET
| Case no. | Age (yr) | Sex | Maximal tumor size (cm) | No. of thymic tumors | Resected lymph nodes | Masaoka clinical stage | T | N | M | Histological grade | Adjuvant therapy | Recurrence of CS (months) | Subsequent metastases | Further therapy | Alive/dead (months postsurgery) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 7 | F | 1 | 1 | 0/0 | I | T1 | NX | M0 | Well-diff | Alive (90) | ||||
| 2 | 13 | M | 11.5 | 2 | 17/17 | IVb | T3 | N3 | M0 | Mod-diff | Persistent | Ba | BA (em) | Dead (22) | |
| 3 | 14 | F | nk | nk | nk | nk | nk | nk | nk | Mod-diff | Yes (36) | c, a, m LN, Ba | S, S, R | Dead (90) | |
| 4 | 17 | M | 6 | 1 | 20/20 | IVb | T3 | N1 | M0 | Mod-diff | Alive (23) | ||||
| 5 | 20 | F | 2 | 1 | 0/1 | IVa | T3 | N0 | M0 | Well-diff | Alive (14) | ||||
| 6 | 21 | M | 5 | 1 | 1/3 | IVb | T2 | N1 | M0 | Mod-diff | R | Yes (24) | nk | C | Dead (72) |
| 7 | 24 | F | 2.5 | 3 | 3/3 | IVb | T2 | N1 | M0 | Mod-diff | Yes (25) | mLN, breast | S, R, C, BA | Alive (58) | |
| 8 | 27 | M | 8 | 1 | 0/0 | III | T3 | NX | M0 | Mod-diff | C, R(G) | Yes (28) | L, mLNa, Ba | C, BA | Dead (56) |
| 9 | 40 | M | 5 | 2 | 2/12 | IVb | T2 | N1 | M0 | Mod-diff | Yes (nk) | mLN | S, BA | Dead (57) | |
| 10 | 50 | M | 2 | 1 | 0/2 | I | T1 | N0 | M0 | Well-diff | Alive (40) | ||||
| 11 | 51 | M | 8 | 1 | 1/1 | IVb | T3 | N1 | M0 | Mod-diff | C, R | Yes (20) | cLN, Ba, La, Par | S, S, BA | Dead (50) |
| 12 | 45 | M | nk | nk | nk | IVb | T3 | N1 | M0 | Mod-diff | C, R | Yes (48)b | c, a, m LN, Ba | R, S, C | Alive (62) |
Age indicates age at time of presentation. Sex: M, male, F, female. Resected lymph nodes indicates the number of lymph nodes positive for NET/number of lymph nodes resected. Masaoka clinical staging: stage I, completely encapsulated tumor; stage II, invasion of tumor into the surrounding tissues; stage III, invasion into surrounding organs such as the pericardium or great vessels; stage IVa, pleural or pericardial invasion; and stage IVb, lymphogenous or hematogenous metastases. Yamakawa-Masaoka TNM staging: T, tumor; T1, completely encapsulated; T2, macroscopic invasion to the surrounding tissue or invasion of the capsule; T3, invasion into neighboring organs; N, nodes; NX, not sampled; N1, metastasis to anterior mediastinal lymph nodes; M, metastasis, M0, no hematogenous metastasis; M1, hematogenous metastasis. Histological grade: well-diff, well-differentiated neuroendocrine carcinoma (low grade, conventional carcinoid); mod-diff, moderately differentiated neuroendocrine carcinoma (intermediate grade, atypical carcinoid). Subsequent metastases: m,c,a LN, mediastinal, cervical, axillary lymph nodes; B, bone; L, liver; Par, parotid gland. Adjuvant and further therapy: S, surgery to remove recurrence of metastasis; R, radiation therapy (adjuvant dose, 54–60 cGy); R(G), radiation therapy with gemcitabine as a radiosensitizer; C, chemotherapy; BA, surgical bilateral adrenalectomy; BA (em), bilateral adrenalectomy by embolization; nk, not known.
Inferred from imaging without pathology.
Patient 12 was first diagnosed with CS 48 months after surgery.